Synonyms
Myosarcoma; Rhabdopoietic sarcoma; Rhabdosarcoma
Definition
Rhabdomyosarcoma (RMS) is a malignant tumor with skeletal muscle differentiation. Four subtypes are currently recognized: embryonal, alveolar, pleomorphic, and spindle cell/sclerosing. The embryonal and the spindle cell/sclerosing subtypes are by far the most common in the urogenital tract.
Clinical Features
Incidence
RMS is the most common pediatric soft tissue sarcoma, and approximately 10–15% of all RMSs originate in the bladder and prostate, making the genitourinary tract the second most common primary site.
Age
RMS is a tumor of childhood and adolescence. Only rare cases have been reported in adults.
Sex
Urinary bladder RMS shows a slight male predominance.
Site
Embryonal RMS occurs in the soft tissue of the paratesticular region, bladder, and prostate. Spindle cell RMS involves the paratesticular region, while alveolar and pleomorphic RMS are exceedingly rare in the urogenital tract.
Treatment...
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References and Further Reading
Ferrari, A., Bisogno, G., Casanova, M., Meazza, C., Piva, L., Cecchetto, G., Zanetti, I., Pilz, T., Mattke, A., Treuner, J., & Carli, M. (2002). Paratesticular rhabdomyosarcoma: Report from the Italian and German Cooperative Group. Journal of Clinical Oncology, 20, 449–455.
Fletcher, C. D., Bridge, J. A., Hogendoorn, P. C. W., & Mertens, F. (Eds.). (2013). WHO classification of tumours of soft tissue and bone. Lyon: IARC Press.
Rodeberg, D. A., Anderson, J. R., Arndt, C. A., Ferrer, F. A., Raney, R. B., Jenney, M. E., Brecht, I. B., Koscielniak, E., Carli, M., Bisogno, G., Oberlin, O., Rey, A., Ullrich, F., Stevens, M. C., & Meyer, W. H. (2011). Comparison of outcomes based on treatment algorithms for rhabdomyosarcoma of the bladder/prostate: Combined results from the Children’s Oncology Group, German Cooperative Soft Tissue Sarcoma Study, Italian Cooperative Group, and International Society of Pediatric Oncology Malignant Mesenchymal Tumors Committee. International Journal of Cancer, 128, 1232–1239.
RodrÃguez, D., Barrisford, G. W., Sanchez, A., Preston, M. A., Kreydin, E. I., & Olumi, A. F. (2014). Primary spermatic cord tumors: Disease characteristics, prognostic factors, and treatment outcomes. Urologic Oncology, 32, 52.e19–52.e25.
Seitz, G., Dantonello, T. M., Int-Veen, C., Blumenstock, G., Godzinski, J., Klingebiel, T., Schuck, A., Leuschner, I., Koscielniak, E., Fuchs, J., & CWS-96 Study Group. (2011). Treatment efficiency, outcome and surgical treatment problems in patients suffering from localized embryonal bladder/prostate rhabdomyosarcoma: A report from the cooperative soft tissue sarcoma trial CWS-96. Pediatric Blood & Cancer, 56, 718–724.
Tavora, F., Kryvenko, O. N., & Epstein, J. I. (2013). Mesenchymal tumours of the bladder and prostate: An update. Pathology, 45, 104–115.
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Franchi, A. (2020). Rhabdomyosarcoma. In: Raspollini, M.R., Lopez-Beltran, A. (eds) Uropathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-030-41894-6_4931
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DOI: https://doi.org/10.1007/978-3-030-41894-6_4931
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